亨廷顿舞蹈症的病程与发病年龄无关。
Duration of illness in Huntington's disease is not related to age at onset.
作者信息
Roos R A, Hermans J, Vegter-van der Vlis M, van Ommen G J, Bruyn G W
机构信息
Department of Neurology, Academic Hospital, Leiden, The Netherlands.
出版信息
J Neurol Neurosurg Psychiatry. 1993 Jan;56(1):98-100. doi: 10.1136/jnnp.56.1.98.
The age at onset and duration of illness were studied in patients with Huntington's disease in the Leiden Roster which at 1 July 1990 contained 2787 patients. Of 1106 patients, 800 deceased and 306 alive, the age at onset was known. The median duration was 16.2 (range 2-45) years. In contrast to the current opinion, the median duration was independent of the age of onset. The median duration in juvenile Huntington's disease was 17.1 years, which is much longer than reported in the literature, and comparable with the categories for the age of onset of 20-34 and 35-49 years. Only in the group where onset was over 50 years of age was the median duration somewhat shorter (15.6 years), which can be ascribed to unrelated causes of death. As age of onset and duration of illness are not related, at least two mechanisms to determine the clinical course have to be postulated: one for age of onset and another for duration of illness. Duration was shorter for males, especially for those with an affected father.
在莱顿登记册中对亨廷顿舞蹈症患者的发病年龄和病程进行了研究，该登记册在1990年7月1日时有2787名患者。在1106名患者中，800名已去世，306名仍在世，其发病年龄已知。病程中位数为16.2年（范围为2至45年）。与目前的观点相反，病程中位数与发病年龄无关。青少年型亨廷顿舞蹈症的病程中位数为17.1年，这比文献报道的要长得多，与发病年龄在20 - 34岁和35 - 49岁的类别相当。只有在发病年龄超过50岁的组中，病程中位数稍短（15.6年），这可归因于无关的死亡原因。由于发病年龄和病程无关，至少必须假定两种决定临床病程的机制：一种用于发病年龄，另一种用于病程。男性的病程较短，尤其是那些父亲患病的男性。
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